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Phenotypes Associated with This Genotype
Genotype
MGI:4880715
Allelic
Composition
Tg(GFAP-cre)8Gtm/0
Tsc2tm1.1Mjg/Tsc2tm1.1Mjg
Genetic
Background
involves: 129X1/SvJ * 129S1/Sv * C57BL/6 * CBA
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tg(GFAP-cre)8Gtm mutation (1 available)
Tsc2tm1.1Mjg mutation (1 available); any Tsc2 mutation (78 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
mortality/aging
• 50% mortality around 7 weeks of age and 100% mortality by 10 weeks
• live shorter than Tsc1tm1Djk/Tsc1tm1Djk Tg(GFAP-cre)8Gtm/0 mice
• prolonged survival after rapamycin treatment

growth/size/body
• poor weight gain
• improved weight gain after rapamycin treatment

nervous system
• seizures at around 2-3 weeks of life
• occasional seizures at 3 weeks of age, earlier onset than Tsc1tm1Djk/Tsc1tm1Djk Tg(GFAP-cre)8Gtm/0 mice
• become progressively more frequent over the ensuing month
• have more seizures than Tsc1tm1Djk/Tsc1tm1Djk Tg(GFAP-cre)8Gtm/0 mice
• fewer seizures after rapamycin treatment
• gross, generalized megencephaly
• rapamycin decreases the megencephaly
• increased brain weight starting at 3 weeks of age
• become progressively more obvious with time
• more severe than Tsc1tm1Djk/Tsc1tm1Djk Tg(GFAP-cre)8Gtm/0 mice
• progressive neuronal disorganization in hippocampus with a dispersion of the pyramidal cell layer
• more severe than Tsc1tm1Djk/Tsc1tm1Djk Tg(GFAP-cre)8Gtm/0 mice
• greater CA1 pyramidal cell layer width in hippocampus than Tsc1tm1Djk/Tsc1tm1Djk Tg(GFAP-cre)8Gtm/0 mice
• rapamycin reduces the dispersion of the pyramidal cell layer in hippocampus
• progressive increase in astrocyte number diffusely throughout the brain
• most obviously in neocortex and hippocampus
• detectable as early as 1 week of life, earlier onset than Tsc1tm1Djk/Tsc1tm1Djk Tg(GFAP-cre)8Gtm/0 mice
• progressively more severe beyond 3 weeks of age
• more severe than Tsc1tm1Djk/Tsc1tm1Djk Tg(GFAP-cre)8Gtm/0 mice
• rapamycin decreases the proliferation of astrocytes

behavior/neurological
• seizures at around 2-3 weeks of life
• occasional seizures at 3 weeks of age, earlier onset than Tsc1tm1Djk/Tsc1tm1Djk Tg(GFAP-cre)8Gtm/0 mice
• become progressively more frequent over the ensuing month
• have more seizures than Tsc1tm1Djk/Tsc1tm1Djk Tg(GFAP-cre)8Gtm/0 mice
• fewer seizures after rapamycin treatment

Mouse Models of Human Disease
DO ID OMIM ID(s) Ref(s)
tuberous sclerosis DOID:13515 OMIM:PS191100
J:167241


Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Models of Human Cancer database (MMHCdb) (formerly Mouse Tumor Biology (MTB)), Gene Ontology (GO)
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last database update
12/10/2024
MGI 6.24
The Jackson Laboratory